原发骨髓淋巴瘤临床特征及预后分析  

Clinical Characteristics Analysis of EBV-associated Hemophagocytic Lymphohistiocytosis Patients with Acute Kidney Injury

作　　者：陈巧琳 冯友繁[1] 伏媛[1] 刘菲 张雯洁[1] 陈阳 魏小芳 张启科[1] CHEN Qiao-Lin;FENG You-Fan;FU Yuan;LIU Fei;ZHANG Wen-Jie;CHEN Yang;WEI Xiao-Fang;ZHANG Qi-Ke(Department of Hematology,Gansu Prowincial Haspital,Lonzhou 730000,Gansu Province,China)

机构地区：[1]甘肃省人民医院血液科,甘肃兰州730000

出　　处：《中国实验血液学杂志》2024年第4期1117-1120,共4页Journal of Experimental Hematology

摘　　要：目的:探讨原发骨髓淋巴瘤患者的临床特点及预后。方法:收集2011年2月至2023年3月甘肃省人民医院收治的6例原发骨髓淋巴瘤患者的临床资料,并对其临床特点及预后进行回顾性分析和总结。结果:6例患者的中位发病年龄为61(52-74)岁;男性2例,女性4例,均有血常规异常,5例患者有发热。体格检查及影像学检查无淋巴结肿大,无肺部、胃肠、肝脾、皮肤等结外病变,排除系统性淋巴瘤累及骨髓,依据骨髓相关检查等确诊,其中5例为原发骨髓弥漫大B细胞淋巴瘤,1例为原发骨髓外周T细胞淋巴瘤(非特指型)。1例放弃治疗,5例给予以CHOP样或联合R的方案,其中1例原发骨髓弥漫大B细胞淋巴瘤免疫化疗达完全缓解后行自体造血干细胞移植。4例死亡,2例存活,中位OS为5.5(1-36)个月。结论:原发骨髓淋巴瘤预后差,骨髓相关检查是确诊的重要手段,以弥漫大B细胞淋巴瘤为主,自体造血干细胞移植可能改善预后。Objective:To analyze the clinical characteristics of patients with Epstein-Barr virus(EBV)-associated hemophagocytic lymphohistiocytosis(HLH)with acute kidney injury(AKI).Methods:EBV-HLH patients who were hospitalized in our hospital from January 2014 to December 2020 were collected,and their clinical characteristics,treatment,concurrent acute kidney injury and prognosis were retrospectively analyzed.Results:In this study,the incidence of AKI complicated by EBV-HLH was 65.5%,and the 28-day mortality rate was 15.3%.Compared with non-AKI group,patients in the AKI group had higher levels of bilirubin,lactate dehydrogenase,creatinine,urea nitrogen,andβ_2-microglobulin(β_2-MG),poorer coagulation,and lower soluble IL-2 receptor(sCD25).Patients in the AKI group had a higher proportion of chemotherapy,transplantation,mechanical ventilation,and the application of vasoactive medications,and were hospitalized for longer periods of time,with higher in-hospital mortality rates and 28-day mortality rates.Patients in the AKI group were analyzed in subgroups according to the Kidney Disease Improving Global Outcomes(KDIGO)classification,and the levels of leukocytes,bilirubin,albumin,creatinine,urea nitrogen,β_2-MG,activated partial thromboplastin time(APTT),and prothrombin time activity(PTA)were more responsive to the severity of the patient′s condition.KDIGO grade 2 and 3 had higher proportions of receiving transplants,diuretics,organ support(mechanical ventilation,application of vasoactive medications,and renal replacement therapy),and admissions to the intensive care unit(ICU),and with higher in-hospital mortality rates and 28-day mortality rates.Regression analysis found that creatinine,β_2-MG,APTT,transplantation,and chemotherapy were independent risk factors for the development of AKI;the application of vasoactive drugs was both an independent risk factor for the development of AKI and for death at 28 days;and chemotherapy,length of hospitalization,and HGB and fibrinogen levels were protective factors for death at28da

关 键 词：原发骨髓淋巴瘤 临床特征 预后 

分 类 号：R733.3[医药卫生—肿瘤]